Epstein-Barr virus-associated autoimmune hemolytic anemia: a clinical report and review of literature

Background: Epstein-Barr virus (EBV) infection is a common disease both in children and adults, but can lead to several complications; involvement of the blood system is often described, particularly neutropenia and thrombocytopenia, but autoimmune hemolytic anemia is rarely seen. Case presentation: A 12-year-old female was admitted to the “G. Di Cristina” Children’s Hospital of Palermo for jaundice and dark urine. Laboratory investigations revealed anemia, increased levels of total and undirect bilirubin, and elevated transaminases, serum lactate dehydrogenase, and reticulocyte count; a peripheral blood smear showed anisocytosis, and the direct antiglobulin test (DAT) for cold agglutinins was positive. The laboratory evaluation of infectious disease showed the presence of EBV VCA IgM and IgG. A diagnosis of acute autoimmune hemolytic anemia EBV related was made: the patient was initially treated with intravenous methylprednisolone and then with intravenous immunoglobulin, which led to a progressive clinical improvement until complete remission. Conclusions: Autoimmune hemolytic anemia is rarely associated with EBV infection; a review of the English literature revealed only 16 cases. Patients with autoimmune hemolytic anemia should always be evaluated for EBV serology, even in the absence of the typical clinical and hematological features of infectious mononucleosis. For these patients, good prognosis is generally expected.